Von willebrand disease vs hemophilia A: 5 Keys differences

Von Willebrand Disease (VWD) and Hemophilia A are both bleeding disorders. They differ in their causes. Their clinical features and management strategies are also distinct.

Causes difference

• Von Willebrand Disease (VWD):
  • This condition is due to a deficiency or dysfunction of von Willebrand factor (vWF). This protein is involved in platelet adhesion and the stabilization of factor VIII.
  • It is the most common inherited bleeding disorder.
• Hemophilia A:
  • Caused by a deficiency or dysfunction of clotting factor VIII, which is essential for the formation of the blood clot.
  • It is an X-linked recessive disorder, primarily affecting males.

Inheritance difference

• VWD:
  • Typically autosomal dominant (most cases), but some severe forms can be autosomal recessive.
  • Affects both males and females equally.
• Hemophilia A:
  • X-linked recessive, primarily affecting males. Females are usually carriers but can have mild symptoms.

Clinical Features difference

• VWD:
  • Mucocutaneous bleeding (e.g., nosebleeds, gum bleeding).
  • Easy bruising.
  • Heavy menstrual bleeding in women (menorrhagia).
  • Prolonged bleeding after surgery or injury.
• Hemophilia A:
  • Deep tissue bleeding (e.g., muscle hematomas, joint bleeds).
  • Spontaneous hemarthroses (bleeding into joints), leading to joint damage over time.
  • Prolonged bleeding after trauma or surgery.

Laboratory Findings difference

• VWD:
  • Prolonged bleeding time.
  • Prolonged partial thromboplastin time (PTT) in some cases (due to factor VIII binding issues).
  • Low vWF levels.
  • Low or normal factor VIII levels.
• Hemophilia A:
  • Prolonged PTT.
  • Normal bleeding time.
  • Low factor VIII levels.

Diagnosis difference

• VWD:
  • vWF antigen levels.
  • vWF activity (ristocetin cofactor assay).
  • Multimer analysis of vWF.
• Hemophilia A:
  • Factor VIII activity assay.

Treatment difference

• VWD:
  • Desmopressin (DDAVP): Increases release of vWF and factor VIII.
  • vWF-containing factor concentrates for severe cases.
  • Antifibrinolytics (e.g., tranexamic acid) for mucosal bleeding.
• Hemophilia A:
  • Recombinant or plasma-derived factor VIII concentrates.
  • Emicizumab (a bispecific antibody mimicking factor VIII) for prophylaxis in severe cases.

Key Differences in Bleeding Patterns

• VWD: Bleeding tends to be mucosal and mild.
• Hemophilia A: Bleeding is often deep, involving muscles and joints, and can be severe.